成人出血型烟雾病的临床特点分析

Analysis of clinical characteristics of 58 adult patients with hemorrhagic moyamoya disease

目的探讨成人出血型烟雾病的临床特点,为提高其诊治水平提供帮助。方法回顾性分析58例成人出血型烟雾病患者的临床资料。结果 58例患者均以中风发病,头颅CT示单纯脑室出血14例、脑室+脑实质出血34例、蛛网膜下腔出血6例、脑实质出血2例、脑实质出血+蛛网膜下腔出血1例。MRI见"常春藤"征者5例、基底节区血管留空影4例。DSA示合并动脉瘤或疑似动脉瘤8例。按GOS评定预后,恢复良好34例,中残15例,重残2例,植物生存1例,死亡6例,31例随访6月至10年,病史大于3年者11例,4例发生再次脑出血;病史小于3年者20例,2例发生再次脑出血。结论脑室出血合并脑实质出血是成年出血型烟雾病的主要出血方式;临床上对于不明原因的无血管病变危险因素的脑出血患者均应常规行脑血管造影检查。

Objectives To study clinical characteristics of the hemorrhagic moyamoya disease （HMD） and to improve its diagnosis and treatment. Methods The clinical data of 58 patients with HMD which was definitely diagnosed by DSA were analyzed retrospectively, including clinical manifestation, imaging characteristics, hemorrhagic type and prognosis. Results All the patients had strokes. CT scan showed that the hemorrhagic types included the ventricular hemorrhage in 14 cases and ventricular hemorrhage accompanied with cerebral parenchymal hemorrhage in 34, subarachnoid hemorrhage （SAH） in 6, cerebral parenchymal hemorrhage in 2 and cerebral parenchymal hemorrhage accompanied with SAH in 1. Aneurysms or suspectable aneurysms were found by DSA in 8 patients. ＂Ivy sign＂ in 5 patients and flow void signal in the basal ganglia regions of 4 patients were found on MRI. Of 58 patients with HMD, 34 were recorded well, 15 were moderately disabled, 2 serverely disabled and 6 died. Thirty-one patients were followed up from 6 months to 10 years. The cerebral haemorrhage recurred in 4 of 11 patients who had HMD for more than 3 years and in 2 of 20 patients who had HMD for less than 3 years. Conclusions The ventricular hemorrhage accompanied with cerebral parenchymal hemorrhage was the major type of intracranial hemorrhage in the adult patients with HMD. The rehaemorrhagic risk significantly increases in patients who had HMD for more than 3 years compared to that in the who had patients HMD for less than 3 years. MRA and CTA examinations are helpful to diagnosis of HMD, which can be definitely diagnosed by DSA.